Sudden death in septo-optic dysplasia: Report of 5 cases

Michael C. Brodsky, Felix A. Conte, David Taylor, Creig S. Hoyt, Robert E. Mrak

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Abstract

Objectives: To report our experience with sudden death in children with septo-optic dysplasia and to identity specific risk factors and suggest preventive measures to minimize mortality. Methods: Clinical data from 5 children with septo-optic dysplasia who died suddenly and unexpectedly were evaluated retrospectively. Results: All children had corticotropin deficiency, all had thermoregulatory disturbances, and 4 children had diabetes insipidus. In at least 4 children, clinical deterioration was caused by lever and dehydration from a presumed viral illness, which appeared to precipitate adrenal crisis. Conclusions: Children with septo-optic dysplasia and hypocortisolism are at risk for sudden death during febrile illness. Thermoregulatory disturbances and dehydration from diabetes insipidus may potentiate clinical deterioration. Prevention of sudden death in septo-optic dysplasia requires early recognition and treatment of these major risk factors.

Original languageEnglish (US)
Pages (from-to)66-70
Number of pages5
JournalArchives of ophthalmology
Volume115
Issue number1
DOIs
StatePublished - Jan 1 1997

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ASJC Scopus subject areas

  • Ophthalmology

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